A rare case of alpha 1-antitrypsin deficiency associated with hypogammaglobulinemia and recurrent pulmonary thrombosis.

Ann Thorac Med

Department of Pulmonology, Allergy and Immunology, St. Luke's Hospital, Chesterfield, MO, USA.

Published: January 2014

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Article Abstract

Alpha 1-antitrypsin (AAT) belongs to the family of serpins (serine protease inhibitors). Loop sheet polymerization is the pathology behind serpinopathies which encompasses AAT, anti-thrombin III and neuroserpin deficiency. To the best of our knowledge, we report the first case of alpha 1-antitrypsin deficiency associated with hypogammaglobulinemia and recurrent pulmonary thrombosis without any concomitant use of drugs.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3912686PMC
http://dx.doi.org/10.4103/1817-1737.124442DOI Listing

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